Alternative Names

Causes, incidence, and risk factors

Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen.

Hemoglobin S changes the red blood cells. The red blood cells become fragile and shaped like crescents or sickles.

The abnormal cells deliver less oxygen to the body's tissues.

They can also easily get stuck in small blood vessels and break into pieces. This can interrupt healthy blood flow and cut down even more on the amount of oxygen flowing to body tissues.

Sickle cell anemia is inherited from both parents. If you get the sickle cell gene from only one parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell anemia.

Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East.

Symptoms

Symptoms usually do not occur until after the age of 4 months.

Almost all people with sickle cell anemia have painful episodes called crises. These can last from hours to days. Crises can cause pain in the lower back, leg, joints, and chest.

Some people have one episode every few years. Others have many episodes each year. The crises can be severe enough to require a hospital stay.

Treatment

The goal of treatment is to manage and control symptoms, and to limit the number of crises. People with sickle cell disease need ongoing treatment, even when not having a crisis. It is best to get care from health care that take care of many patients with sickle cell anemia.

People with this condition should take folic acid supplements. Folic acid helps make new red blood cells.

Treatment for a sickle cell crisis includes:

Blood transfusions (may also be given regularly to prevent stroke)

Pain medicines

Plenty of fluids

Other treatments for sickle cell anemia may include:

Hydroxyurea (Hydrea). Helps reduce the number of pain episodes (including chest pain and breathing problems) in some people

Antibiotics. Help prevent bacterial infections, which are common in children with sickle cell disease

Treatments that may be needed to manage complications of sickle cell anemia include:

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